Lyme Disease: Causes, Symptoms, Staging, Diagnosis, Treatment

Reviewed By: Pramod Kerkar, MD, FFARCSI

What is Lyme Disease?

Lyme disease is a bacterial infection which is passed on to human beings through the bite of deer ticks (Ixodes ticks). Black-legged ticks are responsible for transmitting Lyme disease in the West Coast region.

Where is Lyme Disease Prevalent?

Except for Antarctica, Lyme disease is found all across the United States with a high incidence in the Midwest, East and West Coast regions. This prevalency has significantly increased over the time. Lyme disease is found all over the United States and other countries. It is difficult to diagnose Lyme disease and it can often get misdiagnosed for other conditions. This has led to the belief that the actual number of cases with Lyme disease is higher than what is reported.

Who Gets Affected with Lyme Disease?

People of all ages can get affected with Lyme disease. However, Lyme disease commonly affects older adults, children and people who spend time in outdoor activities, such as park rangers and firefighters.

Why is Lyme Disease Referred as the Great Imitator & Why is it Difficult to Diagnose?

Lyme disease is also referred to as "The Great Imitator," as the symptoms of Lyme disease are similar to many other diseases which make it difficult to diagnose.

Lyme disease is often misdiagnosed as fibromyalgia, chronic fatigue syndrome, multiple sclerosis, and other psychiatric illnesses. This results in delayed treatment which leads to worsening of the underlying infection.

What Part of Body is Affected By Lyme's Disease?

Lyme's disease can affect any organ of the body such as brain, nervous system, heart, joints and muscles.

What Causes Lyme Disease? How Do People Contract Lyme Disease?

The cause of Lyme disease is spirochete, which is a corkscrew-shaped bacterium that goes by the name of Borrelia burgdorferi. Lyme Disease is transmitted through the bite of an immature type of tick which are very tiny, such that even their bite is painless and most of the individuals are not even aware that they are bitten.

After the tick gets attached to the skin and if it is not disturbed, then it can feed for many days. The longer it is attached to the skin the higher are the chances of Lyme disease getting transmitted along with other pathogens into the patient's bloodstream. If pregnant women get infected, then Lyme disease can be passed to their fetus and there are also chances of stillbirth occurring.

How is the Risk of Lyme Disease Predicted?

Not all of the ticks are infected and there is considerable variation in tick infection rates depending on the form of habitat. The variation in the tick infection is about 0% to greater than 70% in the same region. Due to this uncertainty regarding the number of ticks that are infected it makes it difficult to predict the risk of Lyme disease in a region.

What are the Symptoms of Lyme Disease & What are the Stages of Lyme Disease?

Early Lyme Disease & Its Symptoms: The symptoms of early Lyme disease consist of flu-like illness which includes fever, sweats, chills, fatigue, muscle aches, joint pain and nausea. There may also be development of Bell's palsy or a rash. A rash which is shaped like a bull's-eye is thought to be a characteristic feature of Lyme disease, however, there are many patients who develop a different type of Lyme rash or the rash may not be present at all. Around 30 to 80% patients can develop a Lyme disease rash.

Late-Stage or Chronic Lyme Disease & Its Symptoms: If there is delay in diagnosing and treating the Lyme disease, then it becomes late-stage or chronic Lyme disease. The chronic stage of Lyme disease also develops as a result of inadequate treatment. There are some symptoms of late stage or chronic Lyme disease which are similar to symptoms of early stage Lyme disease; however, there are some significant differences.

What is the Importance of Rash as a Symptom in Lyme's Disease?

Many patients will develop a rash which appears as an ordinary red area; however, if the patient develops a "bull's-eye" shaped rash which has a darker edge, then it is a definite sign of Lyme disease and warrants immediate medical attention. Unfortunately the distinctive bull's-eye shaped rash of Lyme's disease develops in less than 10% of patients with Lyme disease.

The rash is mostly diffuse and appears a few days, or in some cases, many weeks after the tick bite. The rash then expands over a period of days or weeks and grows into several inches in width. There may be central clearing in it. If the patient is not treated, then the rash can be present for many weeks before fading or it may subside and then recur. There may be irregularity to the shape of the rash or it can have a scabby or blistering appearance. Some rashes of Lyme disease resemble a bruise. Some of the Lyme rashes look like ringworm, spider bites or cellulitis. There may also be development of multiple "satellite" rashes on different areas of the body.

Why is Lyme Disease Often Misdiagnosed?

Lyme disease can spread to any region of the body and it can affect any system of the body. Lyme disease often affects more than one system of the body. Many symptoms of Lyme disease consist of fatigue, joint pain, cognitive impairment, mood swings, poor sleep, neurological presentations and muscle pain. These symptoms can also be found in other conditions such as chronic fatigue, rheumatoid arthritis, fibromyalgia, depression, Parkinson's disease, multiple sclerosis, ALS and Alzheimer's disease. Due to this, the chances of Lyme disease getting misdiagnosed are high.

What Happens if Lyme's Disease is not Treated?

If there is any delay in the diagnosis and treatment of Lyme's Disease then the spirochetes spread and can go into hiding in various parts of the body. Patient can develop problems within weeks, months and years which involve the brain and nervous system, joints and muscles, heart and blood circulation, reproductive system, digestive system and the skin. The symptoms can subside even without any treatment and patient can experience different symptoms at different times.

Severe symptoms can develop if Lyme's Disease is not treated or is inadequately treated and these symptoms are difficult to resolve. This condition is also known as chronic Lyme disease (CLD) or post-treatment Lyme disease (PTLD).

Which Symptoms of Lyme's Disease are Common With Other Diseases?

The symptoms of Lyme's disease overlap with symptoms in other diseases and some of the common symptoms found in Lyme's disease and other diseases include fatigue, joint pain, sleep impairment, depression, muscle aches, cognitive impairment, headaches and neuropathy. These symptoms may be severe and can severely affect the patient's quality of life.

What is the Quality of Life of Patients with Lyme's Disease?

Many patients who suffer from chronic Lyme disease are greatly debilitated. The patient's quality of life who is suffering from Lyme's disease worsens and is similar to patients who suffer with congestive heart failure. The level of pain experienced in Lyme's disease is similar to that of post-surgical patients. The fatigue experienced in Lyme's disease is equal to the fatigue experienced with multiple sclerosis.

As mentioned before, the patient's quality of life with Lyme's disease is very bad when compared to other chronic illnesses, such as diabetes, heart failure, arthritis and multiple sclerosis. More than half of the patients with chronic Lyme disease are said to have about fair or poor health. Patients suffering from Lyme disease tend to have high disability and unemployment rates.

How is Lyme's Disease Diagnosed?

The diagnostic testing is not reliable in the initial stages of Lyme's Disease and usually gives false negatives. If there is a positive result, then treatment should be started immediately.

A blood sample is taken from the patient who is suspected to suffer from Lyme's disease. Patient's medical history, symptoms and history of exposure to ticks is noted. As the usual diagnostic tests for Lyme's disease are insensitive, a negative result does not necessarily mean that the patient does not have Lyme's disease. There are various reasons why a person will have negative test result despite suffering from Lyme's disease. There may have been delay in development of antibodies; suppression of immune system can be present; or the strain with which the person is infected may not be measured with the test done.

Lyme disease inhibits the immune system and around 20 to 30% of patients will have false negative antibody tests. About half of the patients suffering from chronic Lyme's disease will have a negative ELISA test but a positive Western blot test.

What are the Characteristics of an Ideal Diagnostic Test?

A good test not only helps with diagnosis of the disease, but also helps in managing the condition. A good test helps the physician in assessing the severity of disease, to estimate the prognosis of the patient, monitor the course of progression of disease, its stability, detecting the relapse rate and selecting the medicines and adjusting the treatment. However, such type of test does not exist for Lyme's disease.

What is Two-Tier Testing for Diagnosis of Lyme's Disease?

Indirect type of testing is the commonest test done for diagnosis of Lyme's disease. These tests measure the antibody response of the patient to the infection. The two widely used antibody tests for Lyme's disease are the Western blot and the Enzyme-Linked Immunosorbent Assay (ELISA) test. It is recommended that the ELISA test is done first to screen for the Lyme's disease which is followed by Western blot to confirm Lyme's disease.

These tests, however, are unreliable if done during the initial four-to-six weeks of Lyme infection, as the antibody response is not completely developed in patients. Even in the late stages of Lyme infection, two-tiered testing is still insensitive and misses about half of the patients who have Lyme disease.

How Accurate is Two-Tier Testing for Lyme's Disease? Two-Tier Testing Explained in Detail:

There are 2 tests used in Two-tiered testing. The first one is a screening test which detects a patient having Lyme's disease. The tests which are able to do this well are referred to as having high sensitivity. This test is then followed by a second test which confirms that the patient has Lyme's disease. Tests which are able to achieve this have high specificity.

The second test is highly specific in Lyme disease with only a few false positives. However, the screening test is extremely insensitive and fails to accurately diagnose Lyme's disease. The two-tiered test system misses about half of the patients with Lyme's disease. Because of this, experts recommend to go straight to the Western blot and skip the ELISA test.

What are the Other Tests Done for Diagnosis of Lyme's Disease?

There are 3 more tests which can be done for diagnosis of Lyme disease and these are 1) Polymerase Chain Reaction (PCR); 2) culture testing and 3) antigen detection. These tests are referred to as "direct" tests as they detect the bacteria in the body and not just the immune response to the bacteria.

Polymerase Chain Reaction Test multiplies a vital part of the DNA of Lyme bacteria for its easy detection. However, though PCR is accurate, it also gives many false negatives due to scarcity of the Lyme bacteria and they may not be present in the sample which is tested.

Culture Testing is the "gold standard" test for identification of bacteria. In this test, a sample of blood or other fluids are taken from the patient and growth of Lyme spirochetes is attempted in a special medium.

What is the Treatment for Lyme's Disease?

There are 2 Standards of management for Lyme's disease. The treatment for Lyme disease is controversial. There are two widely divergent views on the best approach for diagnosing and treating Lyme's disease. All this makes it difficult for the patient to get proper diagnosis and get early treatment.

Approach 1: A short course of antibiotics is thought to suffice in treating Lyme disease according to Infectious Diseases Society of America (IDSA). This group also denies that there is any such thing as chronic Lyme disease.

Approach 2: On the contrary, Lyme disease is considered to be difficult to diagnose and difficult to treat by the International Lyme and Associated Diseases Society (ILADS), which results in persistent infection in many patients. Individualized treatment is recommended depending on the severity of symptoms, the presence of other tick-borne co-infections and the response of the patient to treatment.

So, How to Decide the Right Treatment for Lyme's Disease?

It is recommended that both the patient and the doctor make the decision regarding the treatment of Lyme disease. The patient should be given sufficient information regarding the risks and benefits of different treatment options. Then, the doctor and patient should collaborate to arrive at an informed decision that is based on the patient's preferences, beliefs and circumstances.

Treatment for Early Lyme Disease: It is recommended that more aggressive and longer antibiotic course is given to patients in early stages of Lyme Disease. The "high risk" tick bites are treated where the tick was attached for a long time and was not properly removed. The Lyme rash is also treated for a longer period of time so that there is no further progression of disease.

Treatment for Chronic or Late Stage Lyme Disease: It is important that treatment is started early, as earlier the treatment for Lyme Disease, the more successful the result is. However, there is a significant portion of patients who are treated with short-term antibiotics and who continue to experience significant symptoms. Patient's quality of life is also disrupted. The exact cause of these ongoing symptoms is not clear. As of now, there is no test which determines if the patient suffers from active infection or if the infection has been eradicated with the treatment.

Important Points to be Noted for Treating Lyme Disease

It is also thought by the IDSA that the symptoms of Lyme disease occurring after treatment can possibly indicate autoimmune, "post-Lyme syndrome" which does not respond to antibiotics. IDSA uses treatment approaches for acute infections and their treatment guidelines are not recent ones. Whereas, the physicians from ILADS believe that the persistent symptoms may reflect an active infection and treatment should be continued until there is resolution of the symptoms. The treatment approaches used by the physicians are that for persistent infections, such as tuberculosis, where a combination of drugs is used for longer duration of time. The ILADS guidelines have been recently been updated.

What is the Treatment Modality for Lyme's Disease?

The route of administration of antibiotics and the ideal antibiotics, and duration of treatment for persistent Lyme disease is not clearly established. There is no single antibiotic or combination of antibiotics which is capable of completely eradicating the infection. There are reports of relapses and treatment failures with all the current treatment regimens; however, all this is less common if the aggressive treatment is started early.

What are the Risks Associated with Lyme's Disease Treatment?

There are risks associated with all medical treatments. The patient with the doctor should decide on the appropriate treatment for Lyme's disease regarding all the risks and benefits of the treatment.

What are the Factors Which Should Be Considered with Treatment of Lyme's Disease?

Factors to be considered with treatment of Lyme's disease are the possibility of tick-borne co-infections, especially if the patient is having a relapse or if the patient fails to respond to treatment. Other factors which should be considered are immune dysfunction caused by Lyme; silent, opportunistic infections which can occur as a result of immune dysfunction; hormonal imbalances caused by Lyme along with other complications.

What are Other Things Which Should Be Taken into Account with Treatment for Lyme Disease?

The antibiotics given for treatment of Lyme infection wipe out the beneficial intestinal flora which in turn leads to various additional health issues. Therefore, it is important to take probiotics along with antibiotics so that a healthy balance of gut bacteria is maintained. Other than this, antibiotics can also interact with other medicines, food or supplements. All these things should be taken into account before initiating the treatment for Lyme's disease.